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ATP7B

ATP7B(ATPase copper transporting beta),是一种跨膜ATP酶蛋白。它在细胞内负责调节铜元素的转运和代谢。ATP7B的结构包括多个功能区域,其中包括多个转运结构域和催化ATP水解的结构域。这些结构域协同作用,使得ATP7B能够在细胞膜上运输铜离子。 ATP7B主要表达在肝脏细胞的高铜含量亚细胞器中,如高铜含量的高尔基体和胞质溶酶体。它参与了细胞内铜离子的转运和分配,将铜从细胞质输送到高尔基体或从高尔基体释放到胞质溶酶体。 ATP7B的功能对于维持体内铜平衡和避免铜积累至毒性水平非常重要。铜是生命过程中的关键元素,但在高浓度下会对细胞和组织产生毒性作用。因此,ATP7B的异常功能或突变可能导致Wilsons病,这是一种遗传性铜代谢紊乱疾病。对ATP7B的研究对于理解铜代谢的调控机制以及Wilsons病的发病机理非常重要。同时,ATP7B也成为了治疗Wilsons病的一个潜在靶点,研发针对ATP7B的治疗方法有望改善这种疾病的治疗效果。

热销产品

ATP7B Recombinant Monoclonal Antibody CSB-RA175460A0HU

验证数据

IHC
CSB-RA175460A0HU IHC1

IHC image of CSB-RA175460A0HU diluted at 1:50 and staining in paraffin-embedded human brain tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.19% DAB

IHC
CSB-RA175460A0HU IHC2

IHC image of CSB-RA175460A0HU diluted at 1:50 and staining in paraffin-embedded human ovarian cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.19% DAB

IF
CSB-RA175460A0HU IF

Immunofluorescence staining of HepG2 with CSB-RA175460A0HU at 1:20, counter-stained with DAPI. The cells were fixed in 4% formaldehyde and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 494-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).

FC
CSB-RA175460A0HU FC

Overlay Peak curve showing HepG2 cells stained with CSB-RA175460A0HU (red line) at 1:50. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1µg/1*106cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated Goat Anti-rabbit IgG(H+L) at 1:200 dilution for 35min at 4℃.Control antibody (green line) was rabbit IgG (1µg/1*106cells) used under the same conditions. Acquisition of >10,000 events was performed.

ATP7B Antibodies

ATP7B for Homo sapiens (Human)

ATP7B Proteins

ATP7B Proteins for Homo sapiens (Human)

ATP7B Proteins for Rattus norvegicus (Rat)

ATP7B Proteins for Mus musculus (Mouse)

ATP7B Proteins for Ovis aries (Sheep)